ABSTRACT
The last few decades have seen an increasing appreciation of the role of mitochondria in both health and disease. Mitochondria have long been known to play a crucial role in bioenergetics and the production of adenosine triphosphate (ATP) for cellular energy requirements. More recently, mitochondria are seen as an important mediator of intracellular signaling for apoptotic cell death. The last 2 to 3 years have focused on mitochondrial transport and turnover and, in particular, their destruction by autophagy (mitophagy). More than 20 years have passed since the rst description of mutations of mitochondrial (mt) DNA, and in excess of 200 mutations of this molecule are now associated with human diseases. Perhaps the most important discoveries in relation to mitochondrial pathology, at least in terms of patient numbers and disease burden, have been in relation to the neurodegenerative diseases.
